How to cite this article:Chaikhouni A. The magnificent century of cardiothoracic surgery Part 4: Repair of congenital heart defects in the Era of closed heart surgery. Heart Views 2008;9:128-33

How to cite this URL:Chaikhouni A. The magnificent century of cardiothoracic surgery Part 4: Repair of congenital heart defects in the Era of closed heart surgery. Heart Views [serial online] 2008 [cited 2015 Mar 3];9:128-33. Available from: http://www.heartviews.org/text.asp?2008/9/3/128/63765

"For me there have been few things in life, which have been more satisfying than to face a small child struggling for his very existence, to perform some corrective surgical maneuver and later to see the youngster thriving and healthy, starting out in life, sound in body and mind".

Robert Gross

The amazing rise of cardiac surgery erupted in 1896 by the German surgeon Ludwig Rehn (1849-1930) when he successfully repaired a right ventricular stab wound. Supported by great advances in anesthesia and diagnostic methods, closed cardiac operations followed there after with the work of several great pioneers such as: Tuffier, Carrel, Cutler, Souttar, Harken, Bailey, Smithy, and Brock . Surgical approach to the great vessels and some congenital cardiac defects was explored. The saga of patent ductus arteriosus, coarctation of the aorta, and the ingenious methods to close atrial septal defect without cardiopulmonary bypass is another fascinating trip in our tour of the history of cardiothoracic surgery in the Twentieth Century.

Closing patent ductus

The dramatic story of operative closure of patent ductus arteriosus was a clear indicator of the rise of Boston and America in the field of cardiothoracic surgery. As early as 1907, John Munro of Boston suggested the possibility of surgical ligation of patent ductus, and he demonstrated that on a cadaver. However, the first clinical attempt to ligate the ductus was not performed until 30 years later, when John Strieder, also in Boston, attempted operative ligation of an infected patent ductus in 22 year old woman. Unfortunately, the patient died four days later with acute gastric dilatation.

The Father of Modern Cardiac Surgery

After working in the animal lab for about two years, Robert Gross (1905-1988) was able to perform the first successful ligation of a patent ductus on Aug.26th, 1938. This date is considered by many historians as the birthday of modern cardiac surgery. After careful planning and hard work to design and perfect the operation in the experimental lab at Harvard University, the operation was performed at Boston Children's Hospital on a 7 year old girl with patent ductus. Dr. Gross was still chief resident when he carried on this monumental task. However, knowing the objection of his chief William Ladd to the operation, he waited until the chief was on vacation to go ahead with the operation. It is said that Dr. Ladd fired Dr. Gross after his return from vacation! Still, this operation was a major factor in establishing Dr. Gross as an eminent leading figure in cardiac surgery, and was followed by a burst of thousands of such operation all over the World.

The Mother of Pediatric Cardiology

[Additional file 1]Helen Taussig (1898-1986) was appointed In 1930 as head of the new Children's Heart Clinic at the Johns Hopkins Hospital pediatric unit, the Harriet Lane Home, where she worked until her retirement in 1963. She established herself as a leader in developing pediatric cardiology. She suffered from dyslexia, and by the time she graduated from Hopkins in 1927, she had lost her hearing and relied on lip-reading and hearing aids for the rest of her career. Some of her innovations in pediatric cardiology were attributed to her ability to distinguish the rhythms of normal and damaged hearts by touch, rather than by sound. She noticed that patients with cyanotic congenital heart diseases, such as tetralogy of Fallot, deteriorate and die shortly after closure of their useful patent ductus arteriosus. She wondered: What if we can establish some kind of a surgically patent ductus in such patients? Some form of a systemic-pulmonary connection that can deliver more blood into the pulmonary circulation to be oxygenated? Where would she take this original idea about making "a surgical ductus" other than to Dr. Gross in Boston, the "ductus surgeon" of that time. Indeed, she presented this idea to Dr. Gross. However, he was not interested, and said that he had "Enough trouble closing patent ductus without creating one!" Later on, when that idea proved very successful, Dr. Gross remarked that his statement to Helen Taussig was the most stupid statement he ever made in his life.

A Useful Twist in Research Work

Meanwhile,[Additional file 2][Additional file 3] Alfred Blalock (1899-1964) was conducting experiments at Vanderbilt in USA in which he was attempting to produce pulmonary hypertension model in animals. He said: "Sanford Levy and I in 1938 performed operations on dogs in which the proximal end of the severed left subclavian artery was anastomosed to the distal divided end of the left pulmonary artery. In other words, the systemic and pulmonary circulations were connected by a suture anastomosis. Some of these animals were followed for as long as six years after operation. Much to our disappointment arteriosclerosis did not develop …" Three years later, Dr. Blalock was appointed as chairman of the department of surgery at Johns Hopkins University in Baltimore, USA. Dr. Taussig presented her idea about making a surgical patent ductus to Dr. Blalock. By then, he had perfected an end-to-side anastomosis between the left subclavian and the left pulmonary arteries. A new twist in this research was made to change it from developing a model of pulmonary hypertension to a surgical shunt in blue babies. In Nov. 1944, Dr. Blalock performed the first systemic-pulmonary shunt as a palliative procedure to treat cyanotic heart disease. The patient was a 15-month-old girl with a clinical diagnosis of tetralogy of Fallot and severe pulmonary stenosis. At age of 8 months the patient had her first cyanotic spell. Dr. Taussig followed the child for 3 months, and during that time cyanosis increased and she failed to gain weight. She was readmitted, and during the next 6 weeks she refused most of her feedings, lost weight, and weighed only 4 kg at operation. The operation was performed by Dr. Blalock on November 29, 1944. He was assisted by his chief resident William Longmire , and his junior resident, Denton Cooley who administered the I.V. fluids, while Dr. Taussig was watching in the operation theater. The left subclavian artery was anastomosed to the left pulmonary artery in an end-to-side fashion. The postoperative course was stormy, but she was discharged from the hospital 2 months postoperatively. Two additional successful cases were done within 3 months of their first patient. The operation was a successful application of profound understanding of hemodynamics in cyanotic heart disease. It established a new hope for desperately ill babies with previously untreatable cyanotic heart disease. In a very short time, thousands of such operations were performed all over the World, and the names of Blalock and Taussig were all over the medical and popular media.

Who Did it First?

The story of coarctation of the aorta is not as clear. There was some bitter dispute about who did it first. Who did the first successful surgical repair of this disease? Emerging new schools in USA, or the old schools of Europe? There were several claims about "research" work to repair coarctation of the aorta in the early 1940s by Dr. Blalock in Baltimore, Dr. Gross and Dr. Hufnagel in Boston, and Dr. Crafoord in Sweden.

Actually, [Additional file 4] Clarence Crafoord (1899-1984) successfully resected a coarctation of the aorta in a 12-year-old boy on October 19, 1944. Twelve days later he also successfully resected the coarctation of a 27-year-old patient. While Dr. Gross, in Boston, first operated on a 5-year-old boy with this condition on June 28, 1945. After he excised the coarctation and rejoined the aorta, the patient's heart suddenly stopped. The patient died in the operating room. One week later, however, Dr. Gross operated on a second patient, a 12-year-old girl. This patient's operation was successful.

Besides, Charles Hufnagel, who had worked in Dr. Gross's laboratory at the time, wrote: "Gross did not begin experimental work in coarctation before we worked together and probably did not discuss the problem with Crafoord during a visit before the war. We began our experimental studies in the spring of 1944 and I do not believe either group knew of the work or intent of the other" . Crafoord's paper was received at the Journal of Thoracic Surgery on June lst, 1945, about 2 months before Gross even performed such operation. Although there may be some disagreement about two great surgeons' achievements, the credit for solving the coarctation problem should be attributed conjointly to Crafoord, Gross and equally to Hufnageel!

To Open the Heart

By the end of World War II, the diagnostic tools, the basic research, the accumulated clinical experience, and the general mood of the war- experienced enthusiastic surgeons were ready to explore new frontiers. The heart and the great vessels were already approached with success. Dwight Harken (1910-1993) published in 1945 the remarkably successful work of his team in safe removal of many bullets and shrapnel from the heart and great vessels. Surgeons were ready to enter the heart itself to treat diseased valves and to correct congenital heart defects. But how can they approach the heart without inflicting fatal complications? How can they open the heart, support circulation and avoid bleeding, clotting and air embolism? The determined pioneers came up with several successful solutions to these problems.

Blind Feelings Inside the Heart

Various blind cardiac procedures were made to close atrial septal defect (ASD). Some were only applied in animal lab and never made it to clinical application, such as those of Roy Cohn in 1947, Murray, Dodrill, and Swan in 1950s.

Charles Bailey (1910-1993) described atrio-septopexy and applied it in 1952 when he closed ASD by suturing the right atrial (RA) wall of to the edges of the ASD using digital guidance. He used this technique in 21 patients with about 15% mortality.

In 1952, [Additional file 5][Additional file 6] Robert Gross (1905-1988) used a technique called "the atrial well" which is performed by applying a clamp to RA, make an incision, suture an open ended rubber funnel to the edges of the atrial incision and remove the clamp. Blood fills the well to a level equal to the pressure in RA, usually about 5 Cm. The surgeon use digital palpation under the blood pool in the funnel to identify the ASD, the tricuspid valve, the mitral valve and the pulmonary veins. The ASD is usually closed using a patch that is sutured to the edges of the ASD under digital guidance. Despite careful planning and skill, the first three patients died. However, the technique proved useful, and was further developed at Mayo clinic where they reported in 1959 the closure of ASD in 58 patients with no mortality using the atrial well technique. Also, in 1962, Dr. Barrat-Boyes in New Zealand reported the use of this technique in closing ASD with correction of some anomalous pulmonary venous connections in 120 patients with just 2.5% mortality.

Cool them up

Hypothermia was another method which was used to stop the heart and protect the body. In 1950, Wilfred Bigelow in Toronto reported research results on 20 dogs that had been cooled to 20°C, with 15 minutes of circulatory arrest; 11 animals had a cardiotomy, and only 6 animals survived after rewarming. Inspired by the experiments of Bigelow, John Lewis in Minneapolis, USA conducted experiments on hypothermia. On September 2, 1952, Lewis with his first assistant Richard Varco and second assistants, Taufic Mansur and Walton Lillehei, operated on a 5-year-old girl with ASD under general hypothermia and inflow occlusion. The patient was wrapped in refrigerated blankets for about two hours until her rectal temperature fell to 28°C. At this point thoracotomy was performed, and cardiac inflow was occluded for a total of five and one-half minutes. During this time the atrial septal defect was closed under direct vision. The patient was rewarmed by placing her in hot water bath tub at 45°C, and after 35 minutes her rectal temperature had risen to 36°C, at which time she was removed from the bath. Recovery from anesthesia was prompt and her subsequent postoperative course was uneventful. This operation was the world's first successful operation on the open human heart under direct vision, and it marked the beginning of the open-heart surgery era.

Shortly thereafter, Henry Swan at the University of Colorado, USA reported successful results in 13 cases using similar technique. But the use of systemic hypothermia for open intracardiac surgery was relatively short-lived; after the heart-lung machine was introduced clinically, it appeared that deep hypothermia was obsolete. However, during the 1960s it became clear that operative results were poor when cardiopulmonary bypass was used in infants under 1 year of age. Profound hypothermia for cardiac surgery in infants was reintroduced, and the heart-lung machine was used for cooling and rewarming.

200% Mortality!

At the University of Minnesota, [Additional file 7][Additional file 8] Clarence Walton Lillehei (1918-1999) and colleagues studied a technique called controlled cross-circulation. In the animal lab, the circulation of one dog was temporarily used to support that of a second dog while the second dog's heart was temporarily stopped and opened. After a simulated repair in the second dog, the animals were disconnected and allowed to recover.

On March 26th, 1954, Lillehei and his team which included: Richard Varco, Herbert Warden, and Morley Cohen. Dr. Norman Shumway , assistant resident, and Vincent Gott, a surgical intern, were also in the team. They used controlled cross-circulation to correct a ventricular septal defect in a 12-month-old infant. The VSD was closed without a patch during 19 minutes of cross-circulation support; the aorta was not cross-clamped and a cardiotomy sucker was not available for blood retrieval. This child did well for several days but developed pneumonia and died on the 11th postoperative day. One week later, Dr Lillehei and his team successfully closed a VSD in a 4-year-old boy using the same technique. This second patient survived and, at age 48, attended Dr Lillehei's 80th birthday celebration in 1998. Between March 1954 and May 1955, Dr Lillehei operated on 45 children using cross-circulation and became the first surgeon to successfully repair VSD, tetralogy of Fallot, and atrioventricular canal defects. Remarkably, 28 (62%) of these 45 children were discharged from the hospital, and 22 (49%) were alive and well 30 years later. a major cause of hospital mortality in this group of patients undergoing cross-circulation was heart block.

In 1955, when Lillehei and Varco presented their controversial paper on 'cross-circulation' at the American Surgical Association, it was Blalock , the uncontested authority, who opened the discussion in a highly positive manner: "I must say that I never thought I would live to see the day when this type of operative procedure could be performed. I want to commend Drs Lillehei and Varco for their imagination, their courage and industry". On the other hand, Clarence Dennis (Lillehei's senior) thought "that Lillehei was some kind of a nut" . Also, Willis Potts, the noted cardiac surgeon, ironically suggested that the author "… had just presented an operation with a 200% mortality risk" . There is no question that at the time, coming from an as yet unknown surgeon, it was a momentous decision to bring cross-circulation from the laboratory to the operating room. Apparently, Lillehei's experience in World War II battlefields and his own battle with cancer gave him the kind of determination and strength of character to do so. Undoubtedly the stimulation, support and leadership by one of the most important surgical educators, Owen Wangensteen (1898-1981) Chairman of surgery at the University of Minnesota, should not be underestimated.

Heart-Lung Machines

Early in 1950s, there were several programs working hard in a race to develop a machine that can sustain adequate oxygenated perfusion of the whole body to allow safe isolation of the heart and to perform open cardiac procedures under direct vision.

On May 6, 1953, the first successful truly open-heart operation was performed with the use of the heart-lung machine. On that spring day in Philadelphia, John Heysham Gibbon (1903-1973) of the Jefferson University Medical Center, using total cardiopulmonary bypass for 26 minutes, closed a large secundum atrial septal defect in an 18-year-old woman. Beginning with this case, generations of cardiac surgeons have been able to operate on millions of human hearts with good results under direct vision. The story of developing this marvelous machine will be the topic of the next episode in the history of cardiac surgery.[9]